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INTRODUCTION: Desmoid tumors are defined as a rare fibroblastic proliferative disease and may occur in any muscu-loaponeurotic or fascial tissue.
CASE REPORT:A 54-year-old man with FAP referred to our institution presenting fever and abdominal pain. Four yearsbefore a prophylactic colectomy with ileal-rectal anastomosis was done. Three years later a wide intraabdominal masswith desmoid characteristics was detected. In spite of therapy with tamoxifene the RM control showed an increase ofmass dimension. Few days later the patient arrived in our istitute with fever and abdominal pain. Medical therapydidn’t improve clinical symptoms and the patient underwent surgery.
DISCUSSION:Desmoid tumors are benign tumors, but not a benign disease. Most of these tumors occur sporadically, butabout 5% arise in association with familial adenomatous polyposis. Intra-abdominal desmoid tumors remain asympto-matic until their growth and infiltration causes visceral compression. They can cause serious complications and treatmentis often unsuccessful. Aggressive fibromatosis treatment should be evaluated and managed by a multidisciplinary teamwith expertise and experience in sarcoma, prior to initiation of therapy. In FAP, surgery has been recommended forabdominal wall tumors but there are only few indications for intra-abdominal disease like pain, bowel obstruction and desmoids perforation.